DOI: njcpt.com.ng/101.1.2.7
Corresponding author: Abubakar Z. Galadima
Contact email: zaynabgaladima2009@gmail.com
Contact number: +2348036916215
Download Full Manuscript Click Here – A SYSTEMATIC REVIEW OF THE PHARMACOTHERAPY OF VASO-OCCLUSIVE CRISES IN CHILDREN WITH SICKLE CELL ANEMIA
ABSTRACT
BACKGROUND: Annually, over 300,000 children are born with sickle cell disorder (SCD) globally, and Nigeria bears the most significant burden with about 150,000 children. The hallmark of SCD – vasoocclusive crisis (VOC) – predisposes patients to severe pain and shorter life expectancy. There has been no consensus regarding absolute management of VOC, but its alleviation is pertinent to sustaining a good quality of life in children with SCD.
OBJECTIVE: This review aimed to collate and synthesise evidence regarding medications that can be maximised to mitigate VOC in children as modern therapies that have shown promising results over the last five years.
METHOD: A systematic review with two reviewers independently assessed each study against predetermined inclusion-exclusion criteria. Searches were conducted for relevant studies on the pharmacotherapy of VOC within 2016-2021 using PubMed, Google Scholar, and Science-Direct. The selected studies included children between 0- 18years experiencing VOC, who are medicated for prevention, or mitigation of the crises.
RESULT: Twenty-seven unique studies for either prevention or pain control of VOC were reviewed. Forty-eight per cent identified therapies for the prevention of VOC; twenty-six per cent on Hydroxyurea (HU). In addition, Voxoletor and sevapurin were promising therapies with minimal adverse effects. Fifty-two per cent of the studies were on pain control, and Morphine remains the first-line drug in mitigating vaso-occlusive-pain.
CONCLUSION: In the last five years, significant research for promising therapies such as Voxoletor, sevapurin and crizanlizumab to mitigate VOC has improved. However, there remains a significant unmet need in its management. The maximisation of available therapies like hydroxyurea is proven pertinent in the prevention of VOC.
Keywords: vaso-occlusive crisis, sickle cell anaemia, paediatrics, vaso-occlusive pain episodes, pharmacotherapy